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Updated: Jul 10, 2026

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Multicenter comparative study of polysomnography outcomes in children with the monogenic disorder sickle cell disease

Ammar Saadoon Alishlash1, Anis Rabbani Nourani1, Jeffrey Lebensburger1

  • 1Department of Pediatrics, Heersink School of Medicine, University of Alabama at Birmingham, Birmingham, Alabama.

Journal of Clinical Sleep Medicine : JCSM : Official Publication of the American Academy of Sleep Medicine
|September 30, 2024
PubMed

Insights

Sleep-disordered breathing is common in children with sickle cell disease (SCD). This study found significant variations in polysomnography (PSG) outcomes across three US centers, highlighting the need for standardized care.

Area of Science:

  • Pediatric Sleep Medicine
  • Hematology
  • Genetic Disorders

Background:

  • Sleep-disordered breathing (SDB) is a common complication in children with sickle cell disease (SCD).
  • SDB in pediatric SCD patients is linked to adverse health outcomes.
  • Understanding variations in diagnostic procedures like polysomnography (PSG) is crucial for managing SDB in this population.

Purpose of the Study:

  • To compare polysomnography (PSG) outcomes in pediatric patients with sickle cell disease (SCD) across three major US centers.
  • To identify variations in baseline characteristics and PSG findings among these centers.
  • To inform standardized approaches for SDB screening and diagnosis in children with SCD.

Main Methods:

  • A retrospective analysis of 210 pediatric SCD patients (aged 0-21 years) who underwent PSG between 2012 and 2022 at three accredited sleep centers.
  • Descriptive statistics were employed to compare patient demographics, SCD characteristics, and PSG results across the University of Alabama at Birmingham, University of Florida, and Duke University Hospital.
  • Key parameters analyzed included age, BMI, hemoglobin levels, hydroxyurea usage, and specific PSG metrics like apnea-hypopnea index, oxygen saturation, and arousal indices.

Main Results:

  • While baseline characteristics like sex and SCD genotype were comparable, significant differences were observed in age, BMI, mean corpuscular volume, and hydroxyurea usage among the centers.
  • Polysomnography outcomes varied significantly across the three centers.
  • Patients from the University of Florida exhibited worse obstructive sleep apnea and periodic leg movement events, while Duke University Hospital showed higher rates of hypoventilation and arousal.

Conclusions:

  • Multicenter PSG data reveal significant variations in sleep-disordered breathing outcomes for pediatric sickle cell disease patients across different healthcare centers in the southeastern US.
  • These disparities underscore the critical need for standardized protocols in screening, referral for PSG, and interpretation of results for children with SCD.
  • The findings may also be relevant for other genetic disorders with an elevated risk of sleep-disordered breathing.
Abstract

Keywords:
centerscomparisonpediatricpolysomnographypracticesickle cell diseasesleep study

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